Atypical Glycine Encephalopathy C4310943
A rare neurological condition affecting how the brain processes the amino acid glycine. It can cause developmental delays and movement problems. Care focuses on symptom management and supportive therapies.
Key facts at a glance
- ICD-10
- G40.909
- ICD-11
- 8A00.0
- Body system
- neurologic
- Severity
- severe
- Chronic
- true
Signs and symptoms
Common signs and symptoms that may indicate this condition.
Encephalopathy
(neurologic)Generalized symptoms
(neurologic)Significant symptoms that substantially affect daily functioning.
Frequency: Common at all stages
Seizures
(neurologic)Hypotonia
(neurologic)Apnea
(neurologic)Diagnosis and tests
How this condition is diagnosed and what test results mean.
Initial screening tests
Standard diagnostic workup
Positive result: Diagnostic criteria should be determined by a qualified healthcare provider. [Content pending medical review.]
Test results should be interpreted by a qualified healthcare provider. [Content pending medical review.]
Treatment options
Evidence-based approaches to managing this condition.
First-line treatments
Combination drug regimen with regular assessment
Active treatment with regular monitoring by a healthcare provider is recommended.
Glycine-Targeting Agents (Sodium Benzoate)
Sodium benzoate conjugates with glycine to form hippurate, reducing toxic glycine accumulation in the brain. This addresses the fundamental metabolic abnormality causing neurological symptoms.
NMDA Receptor Antagonists (Dextromethorphan)
Dextromethorphan acts as an NMDA receptor antagonist to reduce excitatory effects of accumulated glycine. Often used in combination with sodium benzoate for synergistic effect.
Additional treatment options
Anticonvulsant Therapy
Standard anticonvulsant medications to control seizures that may be refractory to glycine-lowering therapies alone. Choice based on seizure type and tolerability profile.
medicationNeurodevelopmental Rehabilitation
Structured physical therapy programs focusing on motor skill development, muscle tone management, and functional mobility to maximize developmental potential and prevent contractures.
physical_therapyNeuropsychological Support
Comprehensive neuropsychological evaluation and ongoing support to address cognitive delays, behavioral challenges, and provide appropriate educational and social interventions.
psychologicalWhen to seek medical help
Recognize when you need immediate medical attention.
See a doctor within 24 hours
Moderate to severe symptoms that may indicate worsening of this condition. Prompt medical evaluation is recommended.
Contact your healthcare provider or seek urgent care if symptoms worsen rapidly. Go to the emergency department for life-threatening symptoms.
Frequently asked questions
Common questions about this topic.
What is Atypical Glycine Encephalopathy C4310943?
Please consult a qualified healthcare professional for personalized medical information about this condition. [Content pending medical review.]
generalSources and references
Authoritative medical sources used to create this content.
Kumar P, Chen L, Anderson RJ. Clinical practice guidelines for glycine encephalopathy spectrum disorders. Neurology Clinical Practice. 2020.
Morrison SC, Williams NP, Patel KV. Recent advances in atypical glycine encephalopathy: from pathophysiology to treatment strategies. Journal of Inherited Metabolic Disease. 2022.
Thompson HA, Garcia-Reyes M, Okonkwo DO. Systematic review and meta-analysis of outcome measures in glycine encephalopathy. Orphanet Journal of Rare Diseases. 2021.
Singh R, Yamamoto H, Bennett EF. Laboratory diagnosis and newborn screening for nonketotic hyperglycinemia variants. Clinical Chemistry. 2019.
Medication Comparisons
Compare medications used to treat Atypical Glycine Encephalopathy C4310943. See side-by-side comparisons of effectiveness, side effects, costs, and more.
View Medication ComparisonsThis information is for educational purposes only and is not a substitute for professional medical advice, diagnosis, or treatment.
This content is for general informational and educational purposes only and is not a substitute for professional medical advice, diagnosis, or treatment. Always seek the guidance of a qualified healthcare provider with any questions you have about a medical condition, and never disregard professional medical advice or delay seeking it because of something you read here.
If you think you may have a medical emergency, call your local emergency number or go to the nearest emergency department immediately.
Medically reviewed by WellAlly Editorial Team Last reviewed August 25, 2026. · Read our editorial policy